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      Arrhythmogenic Cardiomyopathy: A Review of a Rare Case of Biventricular Phenotype

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          Abstract

          Arrhythmogenic cardiomyopathy is a rare hereditary structural heart disease, with various phenotypes, which mostly affects the right ventricle of the heart, resulting in fibrofatty replacement of the heart muscles and a proclivity to create spontaneous malignant cardiac arrhythmias that may lead to sudden death. Most previous reports were noted on young people. We report a case of its biventricular phenotype in a 61-year-old heavy truck driver who has a current medical history of diabetes mellitus and smoking and was incidentally diagnosed based on the Padua criteria after presenting to the hospital with complaints of lightheadedness and syncope. He eventually had an implantable cardioverter defibrillator, hence preventing death. We were able to correctly diagnose the case and prevent sudden cardiac death by instituting the necessary management.

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          Most cited references45

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          Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria.

          In 1994, an International Task Force proposed criteria for the clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) that facilitated recognition and interpretation of the frequently nonspecific clinical features of ARVC/D. This enabled confirmatory clinical diagnosis in index cases through exclusion of phenocopies and provided a standard on which clinical research and genetic studies could be based. Structural, histological, electrocardiographic, arrhythmic, and familial features of the disease were incorporated into the criteria, subdivided into major and minor categories according to the specificity of their association with ARVC/D. At that time, clinical experience with ARVC/D was dominated by symptomatic index cases and sudden cardiac death victims-the overt or severe end of the disease spectrum. Consequently, the 1994 criteria were highly specific but lacked sensitivity for early and familial disease. Revision of the diagnostic criteria provides guidance on the role of emerging diagnostic modalities and advances in the genetics of ARVC/D. The criteria have been modified to incorporate new knowledge and technology to improve diagnostic sensitivity, but with the important requisite of maintaining diagnostic specificity. The approach of classifying structural, histological, electrocardiographic, arrhythmic, and genetic features of the disease as major and minor criteria has been maintained. In this modification of the Task Force criteria, quantitative criteria are proposed and abnormalities are defined on the basis of comparison with normal subject data. The present modifications of the Task Force Criteria represent a working framework to improve the diagnosis and management of this condition. URL: http://www.clinicaltrials.gov. Unique identifier: NCT00024505.
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            ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): developed in collaboration with the American Association for Thoracic Surgery and Society of Thoracic Surgeons.

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              Diagnosis of arrhythmogenic cardiomyopathy: The Padua criteria

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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                7 October 2022
                October 2022
                : 14
                : 10
                : e30040
                Affiliations
                [1 ] Internal Medicine, Brookdale University Hospital Medical Center, Brooklyn, USA
                [2 ] Cardiology, East Suffolk and North Essex NHS Foundation Trust, Colchester, GBR
                [3 ] Internal Medicine, Maimonides Medical Center, Brooklyn, USA
                [4 ] Internal Medicine, North Mississippi Medical Center, Tupelo, USA
                [5 ] Internal Medicine/Cardiology, Delta State University Teaching Hospital, Oghara, NGA
                Author notes
                Article
                10.7759/cureus.30040
                9637405
                36381856
                50b77e03-51ae-4d33-88b4-0d0a3e4eb4c3
                Copyright © 2022, Aiwuyo et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 7 October 2022
                Categories
                Cardiac/Thoracic/Vascular Surgery
                Cardiology
                Genetics

                sudden cardiac death (scd),heart failure,arrhythmogenic right ventricular cardiomyopathy (arvc/d),biventricular phenotype,arrhythmogenic right ventricular dysplasia

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